Precision medicine in Ewing sarcoma: a translational point of view

• P. Gargallo ^[1] ; A. Juan ^[2] ; Y. Yáñez ^[2] ; S. Dolz ^[1] ; V. Segura ^[2] ; V. Castel ^[2] ; A. Cañete ^[2]
  1. [1] Clinical and Translational Oncology Research Group, La Fe Hospital, Av. Fernando Abril Martorell 106, 46026 Valencia, Spain
  2. [2] Pediatric Hematology and Oncology Unit, La Fe Hospital, Valencia, Spain
• Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 22, Nº. 9, 2020, págs. 1440-1454
• Idioma: inglés
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• Resumen

  • Ewing sarcoma is a rare tumor that arises in bones of children and teenagers but, in 15% of the patients it is presented as a primary soft tissue tumor. Balanced reciprocal chimeric translocation t (11;22)(q24;q12), which encodes an oncogenic protein fusion (EWSR1/FLI1), is the most generalized and characteristic molecular event. Using conventional treatments, (chemotherapy, surgery and radiotherapy) long-term overall survival rate is 30% for patients with disseminated disease and 65–75% for patients with localized tumors. Urgent new effective drug development is a challenge. This review summarizes the preclinical and clinical investigational knowledge about prognostic and targetable biomarkers in Ewing sarcoma, finally suggesting a workflow for precision medicine committees